Dubin-Johnson Syndrome

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Step 1: View clinicals

A 26-year old primigravid woman of 30 weeks gestation presents with worsening non-pruritic jaundice for one week. There is no associated abdominal pain, nausea, vomiting, or fever, and her antenatal period has otherwise been uneventful. Her medical history is unremarkable other than a few episodes of jaundice during her teens, all of which resolved spontaneously after a few days. These were not investigated at that time. She neither smokes nor drinks. A hepatitis panel is negative for hepatitis A IgM antibodies, hepatitis B surface antigen, hepatitis B IgM core antibody, and hepatitis C antibodies.


Step 2: Order all relevant investigations

Complete blood count

WBC/DC: 7,100/mm3 (3,500 - 10,500) Hb: 9.8 g/dL (11 - 18) Hct: 38% (35 - 45) Platelets: 273,000/mm3 (150,000 - 450,000)

Liver function tests

AST: 17 U/L (8 - 48) ALT: 20 U/L (7 - 55) ALP: 68 U/L (45 - 115) GGT: 31 U/L (9 - 48) Total Protein: 7.2 g/dL (6.3 - 7.9) Total Bilirubin: 4.2 mg/dL (0.2 - 1.2) Conjugated Bilirubin: 3.5 mg/dL (0.1 - 0.4)

Ultrasound abdomen

The liver, biliary tree, pancreas, and spleen appear normal. There is an intrauterine pregnancy with a single gestational sac and single fetus and a normal amniotic fluid index. The fetus is compatible with 30 weeks of gestation.

Urine coproporphyrins

Total coproporphyrin (I+III): 180 mcgl/24 hrs (< 200) Coproporphyrin I: 85% (20 - 45%)


Step 3: Select appropriate management

Phenobarbital
Ursodeoxycholic acid
Urgent Cesarean Delivery
Reassurance


Score: ★★☆